Malignant Angioendotheliomatosis: An Angiotropic Lymphoma?

Abstract

A 61-year-old woman developed progressive neurologic deficits and died with pneumonia and septicemia. An autopsy demonstrated the characteristic intravascular and focal perivascular infiltrate of malignant angioendotheliomatosis (MAE) throughout the body but concentrated in the central nervous system and skin. Ultrastructurally, the neoplastic cells lacked evidence of endothelial differentiation. Immunohistochemical studies showed focal staining for Factor VIII–related antigen, probably on a nonspecific basis, negative staining for Ulex europaeus I lectin (an endothelial cell marker), and intense staining for leukocyte common antigen. The authors’ observations provide evidence that at least some examples of MAE are unusual, angiotropic lymphoid neoplasms.